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Canadian Respiratory Journal
Volume 20, Issue 1, Pages e5-e9
http://dx.doi.org/10.1155/2013/406163
Original Article

Twenty-Four Hour Noninvasive Ventilation in Duchenne Muscular Dystrophy: A Safe Alternative to Tracheostomy

Douglas A McKim,1,2 Nadia Griller,3 Carole LeBlanc,4 Andrew Woolnough,4,5 and Judy King6

1Canadian Alternatives in Non-invasive Ventilation Program, Respiratory Rehabilitation Services and The Ottawa Hospital Sleep Centre, Canada
2Department of Medicine, University of Ottawa, Ottawa, Canada
3University of Toronto, Toronto, Canada
4The Ottawa Hospital, Rehabilitation Centre, Canada
5Institute for Rehabilitation Research and Development, Canada
6Physiotherapy Program, School of Rehabilitation Sciences, Faculty of Health Sciences, University of Ottawa, Ottawa, Ontario, Canada

Copyright © 2013 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

BACKGROUND: Almost all patients with Duchenne muscular dystrophy (DMD) eventually develop respiratory failure. Once 24 h ventilation is required, either due to incomplete effectiveness of nocturnal noninvasive ventilation (NIV) or bulbar weakness, it is common practice to recommend invasive tracheostomy ventilation; however, noninvasive daytime mouthpiece ventilation (MPV) as an addition to nocturnal mask ventilation is also an alternative.

METHODS: The authors’ experience with 12 DMD patients who used 24 h NIV with mask NIV at night and MPV during daytime hours is reported.

RESULTS: The mean (± SD) age and vital capacity (VC) at initiation of nocturnal (only) NIV subjects were 17.8±3.5 years and 0.90±0.40 L (21% predicted), respectively; and, at the time of MPV, 19.8±3.4 years and 0.57 L (13.2% predicted), respectively. In clinical practice, carbon dioxide (CO2) levels were measured using different methods: arterial blood gas analysis, transcutaneous partial pressure of CO2 and, predominantly, by end-tidal CO2. While the results suggested improved CO2 levels, these were not frequently confirmed by arterial blood gas measurement. The mean survival on 24 h NIV has been 5.7 years (range 0.17 to 12 years). Of the 12 patients, two deaths occurred after 3.75 and four years, respectively, on MPV; the remaining patients continue on 24 h NIV (range two months to 12 years; mean 5.3 years; median 3.5 years).

CONCLUSIONS: Twenty-four hour NIV should be considered a safe alternative for patients with DMD because its use may obviate the need for tracheostomy in patients with chronic respiratory failure requiring more than nocturnal ventilation alone.